Idiopathic Pulmonary Fibrosis

Idiopathic Pulmonary Fibrosis

pathology:                   Inflammatoryinfiltrate and fibrosis of unknown cause


Aetiology:                    Commonestcause of interstitial lung disease (ILD)

Alsoknown as cryptogenic fibrosing alveolitis (CFA)


Symptoms:                   Dry cough and exertional dyspnoea


Signs:                              Bilateralfine end-inspiratory crackles, clubbing


Investigations:          Clinical,exclude other diagnoses and causes


Treatment:                  Steroids,azathioprine, cyclophosphamide, transplant


Complications:          10%develop lung tumour


Prognosis:                   50%at 5 years

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